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Bile duct cancer

Bile duct cancer (also known as cholangiocarcinoma) is caused by an abnormal and uncontrolled growth of cells in your bile duct.

Bile is a digestive fluid that helps to break down fats. It's made by your liver and stored in your gallbladder. Bile passes from your gallbladder into your small bowel to help break down fatty food when you eat. Bile ducts are tubes that connect your liver and gallbladder to your small bowel.

Bile duct cancer almost always starts in a type of tissue called glandular tissue and is therefore known as an adenocarcinoma. Bile duct cancer is rare; about 1,000 people are diagnosed with it each year in the UK.

Types of bile duct cancer

There are different types of bile duct cancer depending on where it originates.

  • Intrahepatic bile duct cancer is where the cancer starts in the part of the bile duct inside your liver.
  • Extrahepatic bile duct cancer is where the cancer starts in the area of the bile ducts outside your liver.

This factsheet is about extrahepatic bile duct cancer. Intrahepatic bile duct cancer is treated the same way as liver cancer.

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How cancer develops
Cells begin to grow in an uncontrolled way
An image showing the location of the bile duct and surrounding structures

Details

  • Symptoms Symptoms of bile duct cancer

    Symptoms of bile duct cancer include:

    • yellowing of your skin and the whites of your eyes (known as jaundice) – this happens when the tumour blocks the bile duct, which causes bile to build up in your blood and tissues
    • dark urine
    • pale-coloured faeces
    • pain or discomfort in your upper abdomen (tummy)
    • a fever
    • loss of appetite or feeling sick
    • weight loss
    • itchy skin

    These symptoms might be caused by something else other than bile duct cancer. But if you have any of them, contact your GP.

  • Diagnosis Diagnosis of bile duct cancer

    Your GP will ask about your symptoms and examine you. He or she may also ask you about your medical history. Your GP may refer you to an oncologist (a doctor who specialises in cancer care).

    You may have the following tests to confirm your diagnosis.

    • Blood tests can check how well your liver is working.
    • Scans – these may include ultrasound, MRI or CT scans. These are done to check your liver, gallbladder and bile ducts.
    • An endoscopic retrograde cholangio-pancreatography (ERCP) test will take images of your pancreatic duct and your bile duct. This is done using an endoscope (a camera attached to a flexible tube), which your doctor or health professional will pass down through your mouth. You will have an anaesthetic spray to numb your throat and a sedative to relax you.
    • In a percutaneous transhepatic cholangiography (PTC) test, your doctor will pass a fine needle into the right-hand side of your abdomen (tummy). He or she will inject a dye into your bile duct and take an X-ray image. You will need a local anaesthetic for this procedure as well as antibiotics to prevent an infection.
    • A particular type of MRI scan known as magnetic resonance cholangiopancreatography (MRCP) may be done instead of ERCP. This means you won’t have any tubes down your throat as in other tests, and it’s safer (no radiation from X-rays). But it is not possible to perform a biopsy with MRCP.
    • Biopsy – a biopsy is a small sample of tissue. This will be collected from your bile duct and sent to a laboratory for testing to determine the type of cells and if these are benign (not cancerous) or cancerous. This is usually done at the same time as an ERCP or PTC.

    If you're found to have cancer, you may need to have other tests to check if the cancer has spread. The process of finding out the stage of a cancer is called staging. The following tests may be used to diagnose bile duct cancer or to check if the cancer has spread.

    • Contrast enhanced CT scan. You will be given “oral contrast” (a type of dye) to drink to make the CT scan images clearer.
    • An endoscopic ultrasound scan (EUS). In this test an endoscope with an ultrasound probe will be used to check your pancreas and the surrounding tissues. It means that a clearer ultrasound picture can be taken from inside your body.
    • A magnetic resonance (MR) angiogram. Dye will be injected into the main artery leading to your liver to check if the cancer has spread to the blood vessels close to your liver, gallbladder and bile ducts.
    • Laparoscopy. A small cut will be made in your abdomen and a camera attached to a flexible tube will be used to look at your bile duct and surrounding tissues. During the procedure, the doctor might take samples of tissue (biopsy) to see if they are cancerous. You will need a general anaesthetic – this means you will be asleep during the procedure.
  • Treatment Treatment of bile duct cancer

    The main treatment for bile duct cancer is surgery. It’s the only way bile duct cancer can be potentially cured. Other options include procedures to help your symptoms and chemotherapy and radiotherapy. Your treatment will depend on the position and size of the cancer in your bile duct, how far it has spread, and your general health. Your doctor will discuss your treatment options with you.

    Surgery

    Surgery can remove the cancer if it hasn't spread beyond your bile duct. This option isn't always suitable as the bile duct is in an awkward position and it may not be possible to remove all of the cancer.

    There are a number of options for surgery.

    • Removal of your bile ducts. If the cancer hasn’t spread beyond your bile ducts, your surgeon will remove them.
    • Partial removal of your liver. If the cancer has started to spread into your liver, your surgeon will remove the affected part, along with your bile ducts.
    • Whipple's procedure. You might have this if the cancer starts in the bile ducts near your pancreas and small bowel. Your surgeon will remove your bile ducts, part of your stomach, part of your small bowel, part of your pancreas, gall bladder and the surrounding lymph nodes. Lymph nodes are glands that are found throughout your body that are part of your immune system.

    Some surgical procedures can’t cure you but can help with some of your symptoms.

    • Bypass surgery – if it's not possible to remove the cancer, your surgeon may suggest bypass surgery to relieve symptoms of jaundice. He or she will connect the bile duct before the blockage to the part after it. This will allow bile to flow from your liver to your small bowel.
    • Stent – your doctor may insert a stent (a small hollow tube) to help relieve symptoms of jaundice. He or she will fit the stent into your bile duct to keep it open so that bile can drain into your small bowel as it normally would. Alternatively, this procedure may also drain through a small tube into a collecting bag on the outside of your abdomen. This can be done using an ERCP or PTC procedure.

    Non-surgical treatments

    Non-surgical treatments will aim to improve your symptoms and your quality of life. They may include the following.

    • Chemotherapy uses medicines to destroy cancer cells. They are usually injected into a vein but sometimes you may be given chemotherapy as tablets. You may have chemotherapy if surgery isn't possible to treat the cancer or after surgery if the operation couldn’t remove all the cancer. It may also be used if the cancer has come back after treatment. However, doctors are still assessing how effective chemotherapy is in bile duct cancer.
    • Radiotherapy uses radiation to destroy cancer cells. A beam of radiation is targeted on the cancerous cells, which shrinks the tumour. You can have radiotherapy externally from a radiotherapy machine, or internally by placing radioactive material close to the tumour (brachytherapy). Radiotherapy is sometimes used after surgery to destroy any remaining cancer cells. Occasionally, it may also be an option if surgery isn't possible. It can't cure bile duct cancer but it can shrink or slow the growth of the cancer. Chemotherapy and radiotherapy may be given together.

    A less common treatment is photodynamic therapy. This uses a light-sensitive medicine and a laser to destroy cancer cells. Your doctor will inject the medicine into a vein. This will get absorbed by cells around your body – the medicine will enter more cancer cells than healthy cells. He or she will then pass a laser over the cells to activate the drug, which will then destroy the cells.

    However, there’s some uncertainty and conflicting evidence about how safe and effective this treatment is. More research is being done but results have yet to be published. If you’re interested in it, talk to your doctor about what it involves and whether it’s the right option for you.

    You can take part in clinical trials as new treatments constantly become available and need to be assessed. Speak to your doctor for more information about them.

    After treatment for cancer, you will have regular check-ups with your doctor to see if there is any evidence that the cancer has returned. If the cancer has already spread, you will regularly see doctors or specialist nurses to have treatment for any symptoms you might have.

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  • Causes Causes of bile duct cancer

    The exact reasons why you may develop bile duct cancer aren't fully understood at present. However, there are certain factors that can increase your chances of getting it, which include the following.

    • Age. Two-thirds of people diagnosed with bile duct cancer are older than 65.
    • Bile stones. These are similar to gallstones but form inside your liver rather than inside your gallbladder.
    • Ulcerative colitis. This inflammatory bowel disease causes swelling and irritation in your bowel.
    • Primary sclerosing cholangitis. This is a liver condition that causes inflammation of the bile ducts.
    • Choledochal cysts. You can be born with these cysts on your bile ducts.
    • Infection with a parasite called the liver fluke. This is mostly found in South Asia and Africa. See our FAQs for more information.
    • Thorotrast. This is a radioactive substance used from the 1930s to the 1950s in X-ray tests.

    There are other things that may increase your chance of getting bile duct cancer, but doctors are less certain of these. They include having conditions like diabetes, cirrhosis (scarring of the liver), hepatitis B, hepatitis C and fatty liver disease. Smoking tobacco, drinking alcohol, being obese, and being exposed to poisons such as dioxins, nitrosamines and vinyl chloride may also increase your risk.

  • Help and support Help and support

    Being diagnosed with cancer can be distressing for you and your family. An important part of cancer treatment is having support to deal with the emotional aspects as well as the physical symptoms. Specialist cancer doctors and nurses are experts in providing the support you need, and may also visit you at home. If you have more advanced cancer, further support is available to you in hospices or at home, and this is called palliative care.

  • FAQs FAQs

    How would I know if I have been infected by a liver fluke parasite?

    Answer

    It’s unlikely you’ll get liver fluke infection in the UK. It mostly occurs if you’ve visited certain countries and eaten raw fish there. Not all people have symptoms but if you’re infection is severe, you may have abdominal (tummy) pain and diarrhoea.

    Explanation

    The three types of liver fluke parasite that are linked to bile duct cancer are:

    • Clonorchis sinensis. This is present in China, Korea, Taiwan, Russia, Vietnam and Japan.
    • Opisthorchis viverrini. This is found in Thailand, Laos, and Cambodia.
    • Opisthorchis felineus. This is found in eastern Europe and Russia.

    The parasites live in your bile ducts and can cause bile duct cancer.

    You usually get liver fluke parasites from eating raw or poorly cooked fish. You’re unlikely to get this infection in the UK. If you visit parts of the world where the parasite is common and eat raw fish while you’re there, there’s more of a chance. The only other way it can reach areas that it’s not usually found is through imported contaminated fish.

    You may not have any symptoms, but they may occur if your infection is more severe. You may have:

    • abdominal pain
    • a fever
    • feeling sick
    • diarrhoea
    • loss of appetite and indigestion

    If you're concerned that you have, or have had liver fluke infection, speak to your doctor.

    If I have surgery for bile duct cancer, how long will I need to stay in hospital?

    Answer

    It depends on how far the cancer has spread, which of your organs the cancer has affected and what type of surgery you will need.

    Explanation

    If the cancer has spread to a number of areas, more complicated surgery may be needed such as Whipple's surgery. This involves removing your bile duct, part of your stomach, part of your small bowel, part of your pancreas, your gallbladder and surrounding lymph nodes.

    You may need to stay in the intensive care unit for a couple of days after your operation. After that you will continue your stay on a general surgical ward for up to two weeks. Ask your surgeon how long he or she expects you to stay in hospital for your particular treatment.

    If bile duct cancer can't be cured, what will happen?

    Answer

    You will be given palliative treatment, which will treat your symptoms but won’t cure you. This will help you feel better on a day-to-day basis.

    Explanation

    Your doctor might choose treatments, such as chemotherapy, to shrink the cancer, prolong your life, or help ease your symptoms if it can't be cured.

    There are two procedures that your doctor may suggest to relieve any symptoms of jaundice.

    • Inserting a stent – this is a hollow tube, five to 10cm long and is as thick as a ballpoint pen refill. It can open up your bile duct and prevent bile building up. It's usually replaced every three to four months.
    • Bypass surgery – your gallbladder or the unaffected part of your bile duct is joined to your small bowel. It bypasses the blocked area in your bile duct, allowing bile to flow from your liver into your small bowel.

    Your doctor will be able to explain how your treatment will help your symptoms.

  • Resources Resources

    Further information


    Sources

    • Bile duct cancer. American Society of Clinical Oncology. www.cancer.net, published July 2013
    • Bile duct cancer. Macmillan Cancer Support. www.macmillan.org.uk, published 1 January 2013
    • Guidelines for the diagnosis and treatment of cholangiocarcinoma: an update. The British Society of Gastroenterology. www.bsg.org.uk, published 12 August 2012
    • The bile ducts. Cancer Research UK. www.cancerresearchuk.org, published 14 May 2013
    • Cholangiocarcinoma. BMJ Best Practice. bestpractice.bmj.com, published 17 June 2013
    • Risks and causes of bile duct cancer. Cancer Research UK. www.cancerresearchuk.org, published 9 May 2013
    • How is bile duct cancer diagnosed? American Cancer Society. www.cancer.org, published 30 October 2010
    • Extrahepatic bile duct cancer treatment. National Cancer Institute. www.cancer.gov, published 31 October 2013
    • Photodynamic therapy for bile duct cancer. National Institute for Health and Care Excellence (NICE), 2005. www.nice.org.uk
    • Bile duct cancer research. Cancer Research UK. www.cancerresearchuk.org, accessed 15 May 2014
    • Gao F, Bai Y, Ma SR, et al. Systematic review: photodynamic therapy for unresectable cholangiocarcinoma. University of York. Centre for Reviews and Dissemination, 2010. www.crd.york.ac.uk
    • Sripa B, Kaewkes S, Sithithaworn P. Liver fluke induces cholangiocarcinoma. PLos Med 2007; 4(7):e201. doi:10.1371/journal.pmed.0040201
    • Cloncharis FAQs. Centers for Disease Control and Prevention. www.cdc.gov, published 10 January 2012
    • Opithorchis FAQs. Centers for Disease control and Prevention. www.cdc.gov, published 10 January 2012
    • Lim JH. Liver flukes: the malady neglected. Korean J Radiol 2011; 12(3):269–79. doi:10.3348/kjr.2011.12.3.269
    • Smout MJ, Laha T, Mulvenna J. A granulin-like growth factor secreted by the carcinogenic liver fluke pisthorchis viverrini, promotes proliferation of host cells. PLos 2009; 5(10):e1000611. doi:10.1371/journal.ppat.1000611
    • Yossepowitch O, Gotesman T, Dan M. Opisthorchiasis from imported fish. Emerg Infect Dis 2004; 10(12):2122–6. doi:10.3201/eid1012.040410
    • Recovering from whipple's procedure. Cancer Research UK. www.cancerresearchuk.org, published 9 January 2013
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    Reviewed by Natalie Heaton, Bupa Health Information Team, May 2014.

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